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The Brown-Séquard syndrome (BSS) is characterized by ipsilateral spastic weakness and decreased proprioceptive and vibratory sensation below the level of the lesion, and contralateral loss of pain and temperature below the level of the lesion. BSS is typically caused by injury, neoplasm, multiple sclerosis, or spinal cord infarction. BSS after vaccination for diphtheria and tetanus has also been described,1 with additional mild spastic weakness on the side opposite the lesion indicating involvement of the contralateral pyramidal tract. Moreover, a case of BSS after infection with Borrelia burgdorferi responded to antibiotic treatment.2 Until now, the only association of virus infection with BSS is brief mention of an incomplete BSS (weak left leg with an extensor response and decreased sensation over the right leg) after T12-distribution zoster3 and features of BSS with varicella zoster virus (VZV) meningoencephalomyelitis and vasculitis.4 Here we report a case of BSS after reactivation of VZV.
A 33-year-old immunocompetent man experienced malaise, nasal congestion, and bifrontal headache, followed by sharp pain over the left ear, cheek, and eye. A few days later, headache worsened, blisters developed in the left ear, and he was treated with oral acyclovir 800 mg five times daily for 4 days. Two days later, he noted numbness and tingling over the left cheek, arm, and trunk, followed by left-sided numbness and weakness. Despite retreatment by an emergency room physician with oral acyclovir 800 mg five times daily for 10 days, left-sided weakness progressed. He was started on oral prednisone 60 mg daily, tapered over 10 days. He developed left posterior neck pain and headache localized over the left eye. Paresthesias and pain developed in his right hand and leg, and his left leg became stiff. An examiner found spasticity in both legs. Brain, cervical, and thoracic MRIs were normal. The patient was immediately treated with IV acyclovir, 850 mg every 8 hours for 14 days. CSF contained 12 leukocytes, all mononuclear; CSF protein was 48 mg/dL and glucose 45 mg/dL with serum glucose of 85. Neurologic examination revealed a spastic paraparesis with clonus, greater on the left, left-sided loss of vibration and position sense to C6, and right-sided loss of pain and temperature to T9. Deep tendon reflexes were brisk bilaterally, and plantar responses were equivocal. Normal laboratory data included double-stranded DNA, RNP, sn-RNP, SSA/Ro immunoglobulin G (IgG), SSB/La, C3, C4, rheumatoid factor, antiphospholipid panel, homocysteine, NMO, Lyme and mycoplasma antibody, ACE, Venereal Disease Research Laboratory, cytomegalovirus PCR, erythrocyte sedimentation rate, B12, and hepatitis panels. Antinuclear antibodies were elevated at 30 (<7.5) with a speckled pattern.
Three days later, cervical spine MRI showed a T2 hyperintense enhancing lesion at C2-3 (figure). Repeat CSF examination 2 days later revealed 1,300 erythrocytes and 21 leukocytes, 80% mononuclear; CSF protein was 53, glucose 88 with serum glucose of 134; there were no oligoclonal bands, and IgG index was 0.67 (0.2–0.6). CSF also contained both anti-VZV IgM and -IgG antibody, but not VZV DNA, with a reduced serum/CSF IgG ratio consistent with intrathecal synthesis of VZV IgG. Six weeks after onset of disease, headache had resolved and strength was normal on the left. Spasticity, hyperreflexia, and sensory loss to T9 remained.
After geniculate zoster, our patient developed BSS. Although zoster can produce both myelitis5 and spinal cord infarction, the gradual progression of myelopathy over days is more consistent with VZV myelitis than spinal cord infarction due to VZV vasculopathy. The detection of anti-VZV IgG antibody, but not VZV DNA, in CSF at the time of myelopathy parallels virologic findings in other cases of VZV myelitis and vasculopathy, emphasizing the diagnostic usefulness of detecting VZV antibody in CSF in the absence of VZV DNA, particularly since VZV myelopathy can occur without rash.5
The development of VZV myelopathy at a site distant from zoster indicates VZV reactivation from multiple ganglia, i.e., pain and rash in one dermatome followed by neurologic disease without rash in a different dermatome. As long as 50 years ago, the observation that many patients with zoster had pain without rash in dermatomes remote from zoster pain with rash6 suggested the possibility of concurrent VZV reactivation from multiple ganglia. VZV DNA was recently found in saliva of 54 patients with trigeminal, cervical, thoracic, or lumbar-distribution zoster,7 indicating VZV reactivation from geniculate ganglia as well as ganglia corresponding to the site of zoster.
The present report not only indicates that VZV can cause BSS, but also further illustrates simultaneous reactivation of virus from multiple ganglia, in this instance, from the geniculate ganglion with rash and from cervical ganglia without rash.
The authors thank Marina Hoffman for editorial assistance and Cathy Allen for assistance in manuscript preparation.
Supported in part by grants AG06127 and NS32623 to Dr. Gilden from the National Institutes of Health.
Disclosure: The authors report no disclosures.
Received June 26, 2008. Accepted in final form August 18, 2008.
Address correspondence and reprint requests to Dr. D.H. Gilden, Department of Neurology, Mail Stop B182, University of Colorado Denver School of Medicine, 4200 E. 9th Ave., Denver, CO 80262; email@example.com